Expression and diagnostic value of NKX3.1 and NKX2.2 in mesenchymal chondrosarcoma / 中华病理学杂志

Objective: To investigate the immunohistochemical expression of NKX3.1 and NKX2.2 in mesenchymal chondrosarcoma (MC), and to explore the differential diagnostic value of NKX3.1 and NKX2.2 in MC and other types of small round cell malignant tumors. Methods: A total of 12 cases of MC and 97 other small round cell malignant tumors diagnosed in Jinling Hospital, Nanjing University School of Medicine from 2001 to 2020 were collected for NKX3.1 and NKX2.2 immunohistochemical detection. Among them, two kinds of NKX3.1 antibodies [rabbit polyclonal antibody and rabbit monoclonal antibody (EP356)] were used for detection in 12 cases of MC, and one NKX3.1 antibody (rabbit polyclonal antibody) was detected in 97 cases of other small round cell malignant tumors, and the relevant literature was reviewed. Results: The 12 MC patients included 7 females and 5 males, with a mean age of 33 years (14-54 years). Nine cases were from bone and three from soft tissue. Among the 12 MC patients, 8 patients had postoperative recurrence or metastasis, and 3 of them died of tumor recurrence or metastasis. Histologically, 12 cases of MC showed typical bidirectional differentiation.The positive rate of both NKX3.1 antibodies in MC was 12/12, NKX3.1 was focal weakly positive in only one of 12 chondrosarcomas (grade 3), 5 alveolar rhabdomyosarcomas, 5 embryonal rhabdomyosarcomas, and 5 solitary fibrous tumors, respectively. The remaining 70 cases of other small round cell malignant tumors were negative. The positive rates of NKX2.2 in MC, Ewing sarcoma and olfactory neuroblastoma were 12/12, 15/15 and 4/5, respectively. In 12 cases of chondrosarcoma (grade 3), 5 cases of poorly differentiated synovial sarcoma, 5 cases of alveolar rhabdomyosarcoma, and 5 cases of solitary fibrous tumor, NKX2.2 was focally and weakly positive in only one case, respectively, and all the remaining 50 cases of other small round cell malignant tumors were negative. Conclusions: The expression of NKX3.1 and NKX2.2 proteins are significant indicators in the diagnosis of MC, and the combined detection of NKX3.1 and NKX2.2 can help distinguish MC from most other small round cell malignant tumors.

Condrosarcoma mesenquimal en región pectoral, reporte de caso / Mesenchymal chondrosarcoma in the pectoral re-gion, case report

Introducción: El condrosarcoma mesenquimal extraesquelético (CME), corresponde al 1% de todos los tumores malignos de los tejidos blandos. Se localizan principalmente en la región de la cabeza y cuello, sobre todo en la órbita, y en la duramadre del cráneo, seguida de las extremidades inferiores particularmente a nivel del muslo. La ubicación pectoral es rara, motivo de reporte. Reporte de caso: Paciente hombre de 38 años quien desarrolla un nódulo de aproximadamente 2 cm de diámetro localizado en región pectoral derecha con dolor mínimo a la palpación y crecimiento rápido. Dos meses después, al momento la de resección, el nódulo mide 7.5x 6.5 x 3.5 cm, y pesa 106g. Presenta aspecto lobulado, color café grisáceo, al corte es de consistencia cauchosa y superficie blanquecina nodular con áreas centrales de aspecto mineralizado/calcificado. Se procesa 6 cortes de parafina y se diagnostica como condrosarcoma mesenquimal (CM). Evolución: El paciente fue egresado y está en control por consulta externa no ha desarrollado recidivas hasta el momento. Conclusiones: El crecimiento acelerado de una masa de consistencia cartilaginosa se correlacionó en este paciente con la presencia de un condrosarcoma mesenquimal extraesquelético.

Introduction: The extraskeletal mesenchymal chondrosarcoma (ESC) corresponds to 1% of all malignant soft tissue tumors. They are located mainly in the head and neck region, especially in the orbit, and in the dura mater of the skull, followed by the lower extremities, particularly at the level of the thigh. Pectoral location is rare, reason for report. Case report: 38-year-old male patient who developed a nodule approximately 2 cm in diameter located in the right pectoral region with minimal pain on palpation and rapid growth. Two months later, at the time of resection, the nodule measures 7.5 x 6.5 x 3.5 cm, and weighs 106g. It has a lobulated appearance, greyish brown color, when cut it is of a rubbery consistency and a nodular whitish surface with central areas of mineralized / calcified appearance. 6 paraffin sections are processed and diagnosed as mesenchymal chondrosarcoma (CM). Evolution: The patient was discharged and is being monitored by an outpatient clinic. He has not developed recurrences to date. Conclusions: The accelerated growth of a mass of cartilaginous consistency was correlated in this patient with the presence of an extraskeletal mesenchymal chondrosarcoma.

Utility of Transmission Electron Microscopy in Small Round Cell Tumors

Small round cell tumors (SRCTs) are a heterogeneous group of neoplasms composed of small, primitive, and undifferentiated cells sharing similar histology under light microscopy. SRCTs include Ewing sarcoma/peripheral neuroectodermal tumor family tumors, neuroblastoma, desmoplastic SRCT, rhabdomyosarcoma, poorly differentiated round cell synovial sarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, small cell malignant peripheral nerve sheath tumor, and small cell schwannoma. Non-Hodgkin\'s malignant lymphoma, myeloid sarcoma, malignant melanoma, and gastrointestinal stromal tumor may also present as SRCT. The current shift towards immunohistochemistry and cytogenetic molecular techniques for SRCT may be inappropriate because of antigenic overlapping or inconclusive molecular results due to the lack of differentiation of primitive cells and unavailable genetic service or limited moleculocytogenetic experience. Although usage has declined, electron microscopy (EM) remains very useful and shows salient features for the diagnosis of SRCTs. Although EM is not always required, it provides reliability and validity in the diagnosis of SRCT. Here, the ultrastructural characteristics of SRCTs are reviewed and we suggest that EM would be utilized as one of the reliable modalities for the diagnosis of undifferentiated and poorly differentiated SRCTs.

Condrossarcoma mesenquimal extraesquelético em um gato: relato de caso / Extraskeletal mesenchymal chondrosarcoma in a cat: case report

Condrossarcoma mesenquimal extraesquelético (CME) é um neoplasma maligno e raro em animais domésticos. Descreve-se um caso de CME em uma gata que apresentava uma massa firme, branco-amarelada, medindo 18cm de diâmetro, aderida à musculatura do membro pélvico esquerdo. O exame citológico revelou presença de células fusiformes individualizadas pleomórficas e agregados de pequenas células ovais, sem bordas definidas em meio à matriz intercelular amorfa. Devido à impossibilidade de tratamento e ao prognóstico desfavorável, foi realizada eutanásia. Microscopicamente foram observadas células fusiformes indiferenciadas e agregados de células condroides pleomórficas. O diagnóstico de CME foi confirmado pelas técnicas de azul alciano, tricrômico de Masson e pela prova imunoistoquímica, utilizando-se anticorpos antivimentina...

Extraskeletal mesenchymal chondrosarcoma (EMC) is a rare malignant tumor in domestic animals. We described a case of EMC in a cat with a mass measuring 18cm in diameter, yellowish-white and firm attached to the muscles in left hind limb. Cytological examination revealed the presence of individual pleomorphic spindle cells and clusters of small oval cells with no hard edges in the midst of an amorphous intercellular matrix. Due to the impossibility of treatment and poor prognosis, the cat was euthanized. Microscopically undifferentiated spindle cells and clusters of pleomorphic chondroid cells were observed. The CME diagnosis was confirmed with thealcian blue, Masson's trichrome and immunohistochemistry techniques, using antivimentin antibodies...

Primary Extraskeletal Mesenchymal Chondrosarcoma of the Anterior Mediastinum

No abstract available.

Primary extraskeletal mesenchymal chondrosarcoma of the vulva

Extraskeletal chondrosarcoma is rare, making up only 1% of reported chondrosarcoma. We experienced 3 cases of extraskeletal chondrosarcoma, especially in vulva. They were suspected as lipoma of the vulva. The patients had noticed a small but growing mass on their vulva which had been palpated earlier. The masses were excised with a 2 cm resection margin. The final pathological reports confirmed extraskeletal mesenchymal chondrosarcoma (EMC) of the vulva revealing no microscopic lesions on the resection margins. After 24 months of following from the initial diagnosis, the patients remain without evidence of any recurrent. Management of EMC is not well studied due to the rare and variable nature of the disease. However, the surgery, such as we had, is the mainstay of local treatment with studies showing better survival in patients who undergo wide surgical resection. The establishment of adjuvant systemic pharmacotherapy could be expected in the future.

Mesenchymal Chondrosarcoma: A Case Report

Mesenchymal chondrosarcoma is a rare disease with poor prognosis. Treatment including wide or radical excision is very important. Radiotherapy and chemotherapy are additional treatment options, but no conclusive results for their efficacy have been shown until date. Imaging modalities can give important clues for diagnosis and management planning. Angioembolization before surgery could be useful as prophylaxis to control intraoperative bleeding, increasing the likelihood of complete resection.

Chondrosarcome mésenchymateux de l'orbite: à propos d'un cas

"Introduction : Le chondrosarcome mesenchymateux de l'orbite est une tumeur tres rare. Seule une trentaine de cas ont ete publies a ce jour. Cette observation rapporte un cas vu au CHU de Brazzaville. Methode : Une patiente de 33 ans avait consulte notre departement pour une exophtalmie gauche. Resultats : On notait une exophtalmie; un important chemosis et une necrose corneenne complete. Le scanner avait objective une masse orbitaire ovoide; multilobee avec une zone centrale calcifie. L'histopathologie avait mis en evidence un aspect dit ""bi-phasique""; faite d'une mosaique de petites cellules fusiformes associees a des hemangiopericytomes au sein d'une matrice cartilagineuse. Conclusion : Le chondrosarcome mesenchymateux de l'orbite est une tumeur exceptionnelle. Son aspect histopathologique particulier permet aisement de le differentier des autres lesions calcifies de l'orbite."

Reconstrução de couro cabeludo após ressecção de condrossarcoma mesenquimal local / Scalp reconstruction after local mesenchymal chondrosarcoma resection

O condrossarcoma mesenquimal é uma variante rara de condrossarcoma, que raramente atinge os tecidos extraósseos. Este tipo de tumor normalmente ocorre em adultos jovens, sendo altamente agressivo, e tende a apresentar recorrência tardia e metástases a distância. No exame histológico, caracteriza-se por apresentar abundantes células mesenquimais indiferenciadas entre ilhas de células cartilaginosas bem diferenciadas. O tratamento cirúrgico com ressecção completa do tumor é o tratamento padrão para o condrossarcoma mesenquimal. Apresentamos aqui o caso de uma paciente de 56 anos que apresentava um condrossarcoma mesenquimal de grandes proporções na região do couro cabeludo. O diagnóstico da lesão foi tardio e, após o estadiamento da mesma, a paciente foi submetida a ressecção total da neoplasia e reconstrução com retalho de rotação extenso de couro cabeludo e enxertia de pele parcial. No presente estudo, pode-se concluir que ainda não há um tratamento realmente efetivo para o condrossarcoma mesenquimal avançado

Mesenchymal chondrosarcoma is a rare variant of chondrosarcoma, which rarely reaches the extra-osseous tissues. This type of tumor usually occurs in young adults and is highly aggressive, tending to present late recurrence and distant metastases. On histological examination it is characterized by abundant undifferentiated mesenchymal cells between islands of well differentiated cartilage cells. Surgical treatment with complete surgical resection is the standard treatment for mesenchymal chondrosarcoma. Here we present the case of a 56-year-old female patient who had a mesenchymal chondrosarcoma of great proportions in the region of the scalp. The diagnosis was late and after the staging of it, the patient underwent total resection of the tumor and reconstruction with extensive rotation flap of scalp and partial skin graft. In this study we can conclude that as yet there is no really effective treatment for advanced mesenchymal chondrosarcoma

Ultrasonographic findings of mesenchymal chondrosarcoma of the mandible: report of a case

Today, ultrasound imaging is being widely used to assess soft tissue lesions in the maxillofacial region. However, ultrasound investigations of intra-osseous lesions are rare, especially for tumors of the jaws. This report emphasized the capability of this useful imaging modality in identification of the characteristics of malignant conditions involving the bone. Mesenchymal chondrosarcoama, one of the unusual malignant conditions of the jaw, was presented in a young male with significant facial swelling. Different imaging modalities parallel with the histopathologic investigation confirmed the diagnosis. Interestingly, destruction of the bony cortex and new bone formation with a characteristic "sun ray appearance", highly suggestive of sarcomas, was manifested on the ultrasonograph. Thus, this report presented the ultrasonographic features of chondrosarcoma of mandible and considered the ultrasonography to be a useful imaging modality to evaluate intra-osseous jaw lesions.

Extraskeletal Mesenchymal Chondrosarcoma in the Axillary Region: Reports of Two Cases

Extraskeletal mesenchymal chondrosarcomas (EMCs) are relatively uncommon, and a location in the upper extremity, especially in the shoulder or axillary region, is rare. Furthermore, the radiographic findings of EMCs do not show any features that distinguish them from other neoplasms, and therefore, definitive diagnoses are made based on histological features. EMC is an aggressive tumor with a poor prognosis, and requires wide surgical excision. However, its treatment may involve peculiarities such as a difficulty in obtaining a proper surgical margin in the axillary region or shoulder. In this report, the authors present two rare cases of EMCs in the axillary region.

Clinicopathologic and immunohistochemical study of 23 cases of mesenchymal chondrosarcoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic and immunohistochemical features of mesenchymal chondrosarcoma.</p><p><b>METHODS</b>The clinical and histologic features of 23 cases of mesenchymal chondrosarcoma were analyzed. Immunohistochemical study was also performed in 14 of the cases.</p><p><b>RESULTS</b>The age of patients ranged from 12 to 47 years. Fourteen of them occurred in males. Thirteen cases involved the bony skeleton and 5 cases affected the soft tissue. The patients presented with pain and/or swelling. Histologically, the tumor consisted of a mixture of undifferentiated small round cells and hyaline cartilage. Transition between the two components was demonstrated and growth plate-like cartilage was observed. Immunohistochemical study showed that the small round cells were positive for Sox9 (14/14), CD99 (12/14), vimentin (6/14), CD56 (4/14), CD57 (4/14), neuron-specific enolase (3/14) and desmin(1/14). They were negative for Coll-II, S-100 protein, epithelial membrane antigen, pan-cytokeratin, synaptophysin, chromogranin A, CD34 and c-erbB2.</p><p><b>CONCLUSIONS</b>Mesenchymal chondrosarcoma is a rare malignant tumor. Thorough histologic examination, when coupled with immunohistochemical findings, is helpful in arriving at a correct diagnosis.</p>

Primary Spinal Dumbbell-Shaped Mesenchymal Chondrosarcoma Located Intradurally and Extradurally

Mesenchymal chondrosarcomas are rare malignant tumors of the bone and soft tissue. Spinal mesenchymal chondrosarcomas are even rarer and, to the best of our knowledge those that are concomitantly located in the intradural and extradural regions, have never been reported. We report a case of a 25-year-old man with back pain and bilateral progressive weakness of the lower extremities. Magnetic resonance imaging revealed a markedly enhanced dumbbell-shaped mass at the T7 level. The lesion was intradurally located at the left side of the spinal cord, and extended extradurally to the extraforminal space through the T7-8 intervertebral foramen. The tumor was completely excised through a posterior approach. Microscopic examination and immunohistochemical studies confirmed mesenchymal chondrosarcoma. Postoperative radiation therapy and chemotherapy were also performed to prevent local recurrence and metastasis. The patient has been symptom-free for two years after surgery. Herein, we reviewed and discussed the clinical characteristics, treatments, and outcomes of primary intraspinal mesenchymal chondrosarcomas in the literature.

Mesenchymal chondrosarcoma on right mandible: a case report

Mesenchymal chondrosarcoma is very aggressive and represents approximately 1% of all chondrosarcomas. While it affects a very wide age range, the peak frequency is in the second decade of life. It may occur in the head and rib region with a predilection for the maxillofacial skeleton. The small cell undifferentiated component may assume a hemangiopericytoma-like vascular pattern and should be distinguished from hemangiopericytoma. Treatment is en bloc resection, the intended tissue margins of excision should be designed to extend well beyond the actual tumor margin, as mesenchymal chondrosarcomas. Aggressive behavior of mesenchymal chondrosarcoma of the jaw, with a tendency for delayed recurrence and metastasis even many years after treatment. The most frequent site of metastasis was the lung. Here we present 52 years old , female case of mesenchymal chondrosarcoma occurs on Rt. mandible.

Condrosarcoma mesenquimal extraesquelético en un paciente pediátrico: revisión de la literatura a propósito de un caso / Extraskeletal mesenchymal chondrosarcoma in a pediatric patient: a case report

El condrosarcoma mesenquimal extraesquelético pertenece a un grupo de tumores cartilaginosos malignos, que son infrecuentes y habitualmente no presentan compromiso óseo. Se presenta más frecuentemente en la órbita, sistema nervioso central y en los tejidos blandos de los miembros inferiores. El compromiso metastásico es generalmente pulmonar y ganglionar, especialmente en pacientes adultos en quienes se ha demostrado una mayor mortalidad. Este artículo tiene como objetivo presentar el caso de un paciente masculino de 14 años de edad, que ingresó a nuestra institución con una lesión sólida localizada a nivel de los tejidos blandos en el aspecto posterior de la pierna derecha, cuyo estudio histopatológico confirmó un condrosarcoma mesenquimal extraesquelético, junto con una revisión bibliográfica del tema.

The extraskeletal mesenchymal chondrosarcoma belongs to a group of infrequent malignant cartilaginous tumors, which are not obvious mainly in the bone. The most common site of such tumors is within the orbit, the central nervous system and the soft tissues of the lower limbs. The commitment is usually metastatic to the lung and lymph nodes, especially in adult patients, who have shown an increased mortality. The paper aims at presenting the case of male patient aged 14, who was admitted to our institution with a solid lesion located at the level of soft tissues in the posterior aspect of the right leg and whose histopathological study confirmed Extraskeletal mesenchymal chondrosarcoma (CME) and the literature review about the same.

Condrosarcoma mesenquimal extraóseo: reporte de caso / Extraosseus mesenquimal chondrosarcoma: case report

El condrosarcoma mesenquimal extraóseo es una lesión rara que representa menos del 2% de las neoplasias cartilaginosas malignas, histológicamente caracterizada por áreas altamente celulares compuestas por células redondas mesenquimales primitivas alternando con focos de diferenciación cartilaginosa. Siendo una lesión de alto grado histológico, tiende a producir metástasis pulmonares por lo que la sobrevida global no es superior al 26% a 10 años. Se presenta este reporte por ser una lesión infrecuente y por representar un diagnóstico diferencial insospechado.

Clinicopathologic diagnosis of de-differentiated chondrosarcoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic and radiologic features of dedifferentiated chondrosarcoma, focusing on its diagnosis and differential diagnosis.</p><p><b>METHOD</b>Clinical, radiological and pathologic findings of 14 cases of dedifferentiated chondrosarcoma (including biopsy and surgical specimens) were analyzed by hematoxylin and eosin stained sections and immunohistochemistry.</p><p><b>RESULTS</b>The mean age of patients was 52 years. The male-to-female ratio was 9:5. The most common sites of involvement were pelvis, femur and humerus, similar to the conventional chondrosarcoma. Radiologically, they were malignant tumors with dimorphic pattern. Grossly, central chondrosarcomas were more common than those of the peripheral. An essential histological feature of dedifferentiated chondrosarcoma was an abrupt interface between the low-grade cartilaginous tumor and high-grade anaplastic sarcoma. The most common dedifferentiated components were osteosarcoma, malignant fibrous histocytoma and fibrosarcoma. False negative diagnosis and erroneous diagnosis were frequent when only one-time biopsy was available.</p><p><b>CONCLUSIONS</b>Dedifferentiated chondrosarcoma is a rare subtype of chondrosarcoma with poor prognosis, which has different features of clinical manifestation, imaging features and pathological characteristics, compared to conventional chondrosarcoma and chondroblastic osteosarcoma.</p>

Condrosarcoma mesenquimal sinonasal grado tres y carcinosarcoma de glándula Mamaria de un canino: estudios clínico e histopatológico / Sinunasal mesenchymal chondrosarcoma grade 3 and mammary gland carcinosarcoma of canine: clinical and histopathology study

A la Clínica Veterinaria de la Universidad de los Llanos, llegó un canino hembra de 9 años de edad raza Chow Chow, cuyo motivo de consulta fue epistaxis bilateral 4 meses atrás y una masa en la región nasal. Según lo reportado por el propietario, la masa había comenzado a salir 1 mes atrás del día de la consulta. Al examen clínico presentaba una masa subdérmica en la región nasal superior de 2 cm de diámetro. Se realizó una citología de la misma y se encontraron células inflamatorias sin presencia de células neoplásicas. El paciente volvió al mes y la masa con un diámetro de 5cm, estaba ubicada en la región nasal superior y región zigomática. El can presentaba disnea, anorexia, deshidratación del 6 por ciento, secreción mucopurulenta nasal y ocular, midriasis bilateral, reflejo corneal bilateral negativo, aumento de la presión infraocular del ojo derecho. En la glándula mamaria inguinal derecha presentaba una masa de 2cm de diámetro dura y móvil. Se realizó una citología por aspirado con aguja fina, de distintas zonas de las masas, tanto de la nariz, como de la glándula mamaria. Se valoró la citología permitiéndose definir el origen tumoral del proceso. Se realizó biopsia por incisión de la región sinonasal, diagnosticándose tumor mesenquimal maligno grado tres condrosarcoma sinonasal, conformado por células cartilaginosas pleomórficas con anisocariosis. Se realizaron radiografías de la región nasal y del pulmón, encontrándose una zona radiopaca a nivel del seno maxilar con osteolisis del hueso, se evidenciaba la masa de aproximadamente 4 cm de ancho por 2 cm de largo en el lado derecho. En el pulmón no se evidenció metástasis. Después del diagnóstico de histopatología se decide realizar la eutanasia, encontrándose histopatológicamente metástasis en pulmón, confirmación del condrosarcoma mesenquimal y un carcinosarcoma de glándula mamaria.

To the Veterinary Clinic of the Universidad de los Llanos, come a 9 years old Chow Chow female canine, whose consultation reason was a bilateral epistaxis occurred 4 months ago, sudden loss of the vision and a mass in the nasal region of 2cm of diameter approximately, according to the report of the owner, the mass showed up 1 month the day of the consultation. To the clinical exam they were the following abnormal discoveries. Hirsute hair, opaque, presented a subdermic mass approximately in the nasal region superior of 2 cm. diameter that grew to 5 cm. diameter after 1 month of the consultation, located in the nasal region superior and zigomatic region, of hard consistency, the animal had dysnea, bilateral mydriasis, the bilateral corneal reflection was negative, increase of the infraocular pressure of the right eye, the realized test of obstacles in the clinic was negative, that which showed us a blindness. Besides a complete clinical exploration and taking of the advantages of the cytology, she was carried out an aspired with fine needle of different areas of the masses so much neoplastic of the nose and of the region of the mammary gland, being obtained two types of samples. The cytology was evaluated to define origin of the tumors of the process. She was carried out biopsy for incision of the sinunosal region being diagnosed wicked tumor mesenchymal Chondrosarcoma sinunasal that commits bone. After having carried out the autopsy they took samples for histopathology. Being diagnosed mesenchymal chondrosarcoma sinunasal grade 3, with metastasis to lung, and a carcinosarcoma of mammary gland.

Mesenchymal chondrosarcoma on the mandibular body: a case report

Mesenchymal chondrosarcoma is a rare malignant tumor of bone and soft tissue. This aggressive form of chondrosarcoma represents only 3% to 9% of all chondrosarcomas. This neoplasm is characterized by sheets or clusters of undifferentiated spindle or round cells surrounding discrete nodules of well-differentiated cartilage. We experienced a case of mesenchymal chondrosarcoma on mandibular body. Two years ago, the patient had been treated the intrabony cystic lesion on mandiblular left body. At that time, cartilage portion was not detected in the cystic specimen. Two years after cyst enucleation, the recurred large neoplasm in the mandibular left body was noted, and it was diagnosed as 4.5 cm sized mesenchymal chondrosarcoma. The mandibular tumor was widely resected and rigid-plate and cervical musculocutaneous flap were used for reconstruction of resected bone and soft tissues. No complications and recurrence were noted for 6 months postoperatively.